perceptual evaluation and rehabilitation system development for congenital hearing loss

Conditions: Congenital Hearing Loss; Gene Therapy; Speech Perception; Rehabilitation; Reliability and Validity

Summary: this study aims to establish a novel multimodal evaluation and precision rehabilitation system for patients with congenital hearing loss who have received hearing aids, cochlear implantation, or emerging gene therapy. the proposed system will integrate age-stratified difficulty levels, combined with a gamified interaction platform or software designed to assess and train three core perceptual domains: auditory speech perception, music perception, and spatial hearing capabilities.

for congenital sensorineural hearing loss, the traditional treatment methods are hearing aids and cochlear implantation. in recent years, groundbreaking progress has been made in the field of gene therapy for deafness. however, existing assessment materials and tools are not fully applicable to gene therapy patients. in addition, most assessment materials also show other limitations, including a relatively single assessment dimension with minimal gamified interaction and a lack of age-stratified difficulty levels, etc., which pose challenges in objective and comprehensive evaluation, especially for younger children. there is an urgent need to redesign, optimize, and supplement current assessment tools to more accurately reflect post-treatment multidimensional perceptual function, establish a comprehensive evaluation system, and provide targeted rehabilitation guidance. therefore, this study aims to upgrade and develop a novel multimodal evaluation and precision rehabilitation system for patients with congenital hearing loss across different ages and various treatment types. based on the existing assessment materials and software, the proposed system will combine the gamified method and interactive, friendly interface to construct a set of interesting and interactive measurements, integrating auditory speech, music perception, spatial hearing assessment and rehabilitation training. patients with congenital hearing loss and healthy controls will be recruited to validate the reliability and validity of the evaluation system and the effectiveness of the rehabilitation training, providing reference for clinical application.

Eligibility criteria:
inclusion criteria: for congenital deafness group: patients with congenital hearing loss with hearing thresholds ≥65 db who have received hearing aids, cochlear implantation, or gene therapy; age between 1 and 35 years old, regardless of gender. for healthy controls group: participants with normal hearing thresholds (≤20 db) whose age and gender matched to the patient group. mandarin chinese as the native language. participants and/or their guardians must provide informed consent before the trial, voluntarily sign a written consent form, and commit to receive evaluation at specified time points. capable of effective communication with researchers under the guardian's assistance and willing to cooperate and comply with the researchers' requirements. participants and/or their guardians should have a correct understanding of the trial and appropriate expectations regarding potential benefits. exclusion criteria: presence of other otological disorders that may interfere with the surgical outcome or interpretation of study endpoints, such as otitis media, meniere's disease, etc. presence of other severe congenital diseases, such as congenital heart disease. presence of severe systemic diseases or in the acute onset of diseases, such as pulmonary tuberculosis, active hepatitis b or c infection, active herpes zoster infection, pancreatitis, renal insufficiency, etc. individuals with low immunity, a history of immune deficiency or organ transplantation. individuals with a history of neurological, mental disorders, or moderate-to-severe cognitive dysfunction, such as epilepsy, dementia, autism spectrum disorders, etc. any other conditions for which the investigators consider the subject unsuitable for participation in this clinical study.

Keywords: congenital hearing loss gene therapy multimodal assessment auditory speech perception music perception spatial hearing rehabilitation